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Desmoplakin cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy: two distinct forms of cardiomyopathy?

Minerva cardiology and angiology - 1 Apr 2022

Kukavica Deni, Trancuccio Alessandro, Arnò Carlo, Latini Alessia C, Mazzanti Andrea, Priori Silvia G

Abstract excerpt

The confirmation of a hypothesis that desmoplakin-related (DSP) cardiomyopathy could represent a distinct clinical entity from the classical, RV-dominant, form of arrhythmogenic cardiomyopathy (ACM), most frequently caused by PKP2 mutations, would without any shadow of doubt signify a turning point in the history of this disease. The concept of gene-specific diseases underneath the umbrella diagnosis of ACM would...

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PMID: 34338490
DOI: 10.23736/S2724-5683.21.05804-X

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